ABSTRACT
Background: Extracranial Germ cell tumors [GCT] are a rare and a heterogeneous group of pediatric cancers but highly curable
Aim: We aimed to review management, outcome and prognostic factors that influence overall survival [OS] in a pediatric Tunisian oncologic unit
Methods: We retrospectively evaluated between January 1998 and December 2012, 33 patients affected by extracranial germ cell tumors and treated according to TGM95 protocol established by the SFOPin a pediatric Tunisian oncologic unit
Results: Patients had a mean age of 57 months [ranges: 1 day-13 years]. There were 19 girls and 14 boys. Primary sites included 12 sacrococcygeal, 11 ovarian, 6 testicular, 3retro peritoneal and 1 mediastinal site. After a mean follow up of 26.1 months [ranges: 0-96 months], OSat 2 years and 5 years were respectively 82% and 75%. Event-free survival were respectively 79% at 2 years and 74% at 5 years. Various prognostic factors have been studied according to Kaplan-Meier. Univariate analyses identified significant factors which influence strongly OS: the stage [p=0.04], the completeness of surgery [p<0.001] and the relapse [p = 0, 0001]. A multivariate study showed that only the quality of resection and the clinical stage remained strong significant prognostic factors [p=0,021] for 5-year OS
Conclusion: Disease stage, completeness of surgery and relapse have been established as the most powerful prognostic parameter in our analysis. The improvement of survival of patients affected by extracranial germ cell tumors in Tunisia is a real achievement mainly due to the success of salvage treatments
ABSTRACT
Soft tissue chondroma is a rare benign tumour, which is generally seen in adult. It consists of islands of heterotopic cartilaginous tissue and most localised on the hands and the feet. The hypothesis that microtrauma is involved in the aetiology of this condition has yet to find any factual support. To report two paediatric cases of soft tissue chondroma. The first is a soft tissue chondroma of the posterior aspect of the left axilla in a 3-year-old boy. CT scans showed a spherical fatty density soft tissue mass without evident calcifications, attaching the infraspinous muscle. The second patient is a 9-year-old girl presented with a right auricular finger soft tissue chondroma. Radiographs showed several punctuated calcifications with adjacent bone scalloping. MRI revealed a lobulated soft tissue mass attaching the flexor tendons. The tumours were entirely removed. Histological examination showed cartilaginous tissue in both cases. At follow-up, the patients had good functions without evidence of recurrence. Simple excision should suffice to treat soft tissue chondroma but care should be taken to make the excision complete if recurrence are to be avoided
Subject(s)
Humans , Male , Female , Child, Preschool , Child , Chondroma/diagnosis , Chondroma/surgeryABSTRACT
Granulocytic sarcoma is a rare malignant extramedullary neoplasm of myeloid precursor cells, occuring before or after onset of leukaemia. Involvment of the head and neck region is rare, generally concerning the orbit. To illustrate imaging findings of granulocytic sarcoma in an unusual location; maxillary sinus. We report a case of maxillary sinus granulocytic sarcoma in a 13-month old boy revealed by facial nerve palsy, ptosis and jugal swelling, without any evidence of haematological disorders. The patient underwent computed tomography and magnetic resonance imaging exam which demonstrated a non specific maxillary sinus mass with destruction of the orbit floor and the alveolar bone. Diagnosis was obtained after bone biopsy allowing histological and immunohistochemical studies. Granulocytic sarcoma is a serious condition because of the associated hematologic disorders. In a sinus or orbit location, imaging features are non specific. Unless hematologic history is present, diagnosis is difficult and an immuno histo chemical study is required
Subject(s)
Humans , Male , Maxillary Sinus , Maxillary Sinus Neoplasms , Facial Paralysis , BlepharoptosisABSTRACT
The aim of this retrospective study is to report eight cases of blunt duodeno-pancreatic trauma in infants, emphasizing on the role of imaging in acute assessment of the lesions and in further management. We reported eight cases of duodeno-pancreatic injuries between 2006 and 2008, 5 boys and 3 girls with an age ranging from 3 to 12 years [median age:7 years]. Trauma circumstances were: car accident [n=2], domestic injury [n=5] and bicycle's fall injury [n=1]. All patients underwent abdominal ultrasonography and CT scan in the initial evaluation and during the follow-up. Imaging showed the following pancreatic lesions: 3 corporeal fractures, 2 caudal fractures and one between the corporeal and the caudal portions. Four pancreatic haematomas were found. Two isolated duodenal haematomas were found. Two patients improved spontaneously, the six others developed complications: 4 retroperitoneal collection. Management was chirurgical in one case medical in two cases, endoscopic in 2 cases and three percutaneaous drainages were performed. Blunt duodeno-pancreatic injuries in children have to be evaluated by an early imaging modality, in order to perform acute assessment of the lesions. Primary conservative treatment is advocated while clinic, biologic and imaging follow-up is required to detect complications, which management can be endoscopic percutaneous or surgical
ABSTRACT
Lipomatous tumours of the limbs in children are rare, and lipoblastoma is the most common soft tissue tumour. Most of them have typical imaging features, but their clinical presentation and their management may vary, depending on the exact histological subtype.The aim of our study is to illustrate the main clinical, radiological and histological features of the different benign lipomatous tumours in children. review of the literature. it is about a descriptive study of paediatric cases of benign lipomatous tumours of limb in children. The imaging findings are helpful and can provide essential components for the diagnosis
Subject(s)
Humans , Male , Female , Extremities , Lipoma/diagnostic imaging , Magnetic Resonance Imaging , Review Literature as Topic , Tomography, X-Ray Computed , Soft Tissue Neoplasms , ChildABSTRACT
Xanthogranulomatous pyelonephritis [XGP] is a specific form of chronic inflammatory kidney disease rarely seen in children. The Symptoms are often vague and non-specific the aim of this paper is to return the particularities of imaging features in xanthogranulomatous pyelonephritis. insisting on differential diagnosis with renal tumors, especially in case of no renal stone or tract obstruction evidence, We report a case of xanthogranulomatous pyelonephritis in a 2-year-old boy involving the lower renal pole which demonstrates the diagnostic difficulties encountered in this disease Xanthogranulomatous pyelonephritis is a rare condition in children and should be included in the differential diagnosis of a child presenting a renal mass
Subject(s)
Humans , Male , Child , Review Literature as Topic , Tomography, X-Ray ComputedSubject(s)
Humans , Male , Female , Orbital Neoplasms/diagnosis , Tomography, X-Ray Computed , Magnetic Resonance ImagingABSTRACT
Involvement of the gastrointestinal tract is frequently reported among the extranodaI sites of non-Hodgkin's lymphoma, but primary lymphoma of the pancreas is very rare. We report the case of an 11-year-old boy explored for epigastric pain with fever leading to the diagnosis of high-grade primary non Hodgkin's T-cell lymphoma originating from pancreas. This pancreatic lesion is documented by sonography, CT and MRI. This patient died quickly with severe infection
Subject(s)
Humans , Male , Pancreatic Neoplasms , Child , Ultrasonography , Tomography, X-Ray Computed , Magnetic Resonance ImagingABSTRACT
We have curried out a retrospective study on 51 children aged between 2 years 3 month and 13 years in order to determine the findings and to define the indications of neuroimaging modalities in children with a first unprovoked seizure. Children who had neuroimaging studies were divided into two groups based on the results of neuroimaging normal or abnormal. We have compared the two groups according to each clinical and electroencephalographic parameters studied. Neuroimaging abnormalitie were found in 47% of cases. According to our results and literature review, we concluded that the imaging should be done if we have at least one of the criteria: an abnormal neurological examination after the seizure and focal slowing waves on the electroencephalogram
Subject(s)
Humans , Male , Female , Child , Retrospective Studies , Tomography, X-Ray Computed , Magnetic Resonance ImagingABSTRACT
Association of hypertrophic osteoarthropathy and malignant thymoma is very rare in children. Authors report a case of a 14 year old girl followed for rheumatic fever since she was 8 years old. She was explored for arthralgia. The exam showed Hippocratic fingers, soft tissues hypertrophy of lower limbs and dyspnoea. Plain radiography had demonstrated symmetrical ossifying periostisis in tubular bones of lower limbs evocating hypertrophic osteoarthropathy diagnosis. Chest radiography has demonstrated bilobate anterior mediastinal mass. Ultrasonography and MRI revealed a voluminous anterior mediastinal mass with cystic and fleshy components. Thymic adenocarcinoma diagnosis was made by surgical biopsy